Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome

• Published in: The Lancet (British edition) Vol. 362; no. 9394; pp. 1457 - 1459
• Main Authors: Behr, E, Wood, DA, Wright, M, Syrris, P, Sheppard, MN, Casey, A, Davies, MJ, McKenna, W
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.11.2003; Elsevier Limited
• Subjects: Adolescent; Adult; Age; Arrhythmias, Cardiac - epidemiology; Arrhythmias, Cardiac - genetics; Arrhythmias, Cardiac - mortality; Cardiomyopathy; Cardiovascular disease; Child; Comorbidity; Coronary artery disease; Death & dying; Death, Sudden, Cardiac - epidemiology; Death, Sudden, Cardiac - etiology; Deoxyribonucleic acid; Disease; DNA; Dystrophy; Echocardiography; Electrocardiography; England - epidemiology; Epidemiology; Exercise Test; Families & family life; Family; Female; Genetic testing; Heart diseases; Heart Diseases - diagnosis; Heart Diseases - epidemiology; Heart Diseases - genetics; Heredity; Hospitals; Humans; Hypotheses; Incidence; Long QT syndrome; Male; Medical diagnosis; Middle Aged; Mortality; Mutation; Myotonic dystrophy; Pathology; Physical Examination; England; United Kingdom > UK
• ISSN: 0140-6736; 1474-547X
• DOI: 10.1016/S0140-6736(03)14692-2

4·1% of sudden cardiac deaths in the 16–64 age-group are unexplained. In this group, cardiac pathological findings are normal and toxicological tests are negative; termed sudden arrythmic death syndrome (SADS). We searched for evidence of inherited cardiac disease in cases of SADS. Of 147 first-degree relatives of 32 people who died of SADS, 109 (74%) underwent cardiological assessment. Seven (22%) of the 32 families were diagnosed with inherited cardiac disease: four with long QT syndrome; one with non-structural cardiac electrophysiological disease; one with myotonic dystrophy; and one with hypertrophic cardiomyopathy. Families of people who die of SADS should be offered assessment in centres with experience of inherited cardiac disease.